Pratama Andi Ahmad Thoriq, Atmaja M Hidayat Surya
Department of Radiology, Dr. Soetomo General Academic Hospital, Surabaya, Indonesia.
Department of Radiology, Faculty of Medicine - Universitas Airlangga, Surabaya, Indonesia.
Radiol Case Rep. 2024 Sep 7;19(12):5605-5611. doi: 10.1016/j.radcr.2024.08.037. eCollection 2024 Dec.
Pelvicoabdominal plexiform neurofibroma is a rare and complicated form of type 1 neurofibromatosis (NF1), distinguished by developing benign nerve sheath tumors in the pelvis and abdomen. A male patient, aged 26, came to our center with dysuria, abdominal bloating, rectal mucosa prolapses, and trouble walking and moving legs. Physical examination revealed a palpable mass of solid consistency fixed in the pelvic cavity to the abdominal cavity. A large and extensive mass in the pelvic to the abdominal region can be evaluated with multimodality radiological imaging, including ultrasound, computed tomography, and magnetic resonance imaging. Imaging is crucial for diagnosis, evaluation of extension, and early detection of potential malignant transformation in these patients. The patient was scheduled for palliative surgical resection due to the extensive mass; however, he did not survive while waiting for the operation. Pathology examination and immunohistochemical staining revealed positive S-100 protein, indicating the neural crest originate lesion. We report the clinical and radiological features of plexiform neurofibroma in a young male patient, confirmed by pathology examination.
盆腔腹部丛状神经纤维瘤是1型神经纤维瘤病(NF1)的一种罕见且复杂的形式,其特征是在盆腔和腹部发生良性神经鞘瘤。一名26岁男性患者因排尿困难、腹胀、直肠黏膜脱垂以及行走和腿部活动困难前来我院。体格检查发现盆腔至腹腔内可触及一个质地坚实的肿块。盆腔至腹部区域的巨大广泛肿块可通过多种影像学检查进行评估,包括超声、计算机断层扫描和磁共振成像。影像学检查对于这些患者的诊断、范围评估以及潜在恶性转化的早期检测至关重要。由于肿块广泛,该患者计划接受姑息性手术切除;然而,他在等待手术期间死亡。病理检查和免疫组化染色显示S-100蛋白阳性,提示神经嵴起源的病变。我们报告了一名年轻男性患者丛状神经纤维瘤的临床和影像学特征,并经病理检查证实。
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